Mucoid Colonic Carcinoma as an Autosomal-Dominant Inherited Syndrome

• An autosomal-dominant mucoid colonic carcinoma syndrome is described. Seventeen members of one family over three generations have experienced cancers, including 14 with adenocarcinoma of the colon. The defective autosomal locus is pleiotropic, producing adenocarcinoma of the endometrium, atypical endometrial hyperplasia, uterine leiomyosarcoma, and bladder transitional and renal cell carcinoma in addition to the mucoid variant of colon carcinoma. ( Arch Surg 1981;116:901-905)